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The pathogenesis of pulmonary fibrosis: a moving target

Wim A Wuyts, C Agostini, KM Antoniou, D Bouros, RC Chambers, V Cottin, JJ Egan, BN Lambrecht, R Lories, H Parfrey, A Prasse, C Robalo-Cordeiro, E Verbeken, JA Verschakelen, AU Wells, GM Verleden
European Respiratory Journal 2012; DOI: 10.1183/09031936.00073012
Wim A Wuyts
*Laboratory of Pneumology, Dept. of Respiratory Medicine; Katholieke Universiteit Leuven and University Hospitals Leuven; Leuven, Belgium
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  • For correspondence: wim.wuyts@uzleuven.be
C Agostini
#Dept of Clinical & Experimental Medicine, Clinical Immunology, Padua University, Italy
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KM Antoniou
¶MD, PhD Lecturer in Thoracic Medicine Medical School, University of Crete, Heraklion, Greece
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D Bouros
+Dept of Pneumonology, Medical School, Democritus University of Thracev Alexandroupolis, Greece
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RC Chambers
§Centre for Inflammation and Tissue Repair, University College London, United Kingdom
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V Cottin
fHospices civils de Lyon, Hôpital Louis Pradel, Service de Pneumologie – Centre de Référence des Maladies Pulmonaires Rares; Université Lyon I; Lyon, F-69677, France
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JJ Egan
**FRCP FRCPI Mater Misericordiae University Hospital St Vincent's University Hospital University College Dublin Ireland
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BN Lambrecht
##Dept of respiratory medicine UZ Gent, Gent Belgium
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R Lories
¶¶MD PhD Associate Professor Laboratory for Skeletal Development and Joint Disorders Division of Rheumatology, Dep. of Musculoskeletal Sciences Katholieke Universiteit Leuven
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H Parfrey
++PhD, FRCP Respiratory Medicine Division, Dept of Medicine, University of Cambridge and Papworth Hospital,Cambridge UK
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A Prasse
§§University Medical Center Freiburg, Dept of Pneumology
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C Robalo-Cordeiro
ffDept of Pulmonology, University Hospital of Coimbra 3000 Coimbra, Portugal
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E Verbeken
***dept of pathology UZ Leuven, Leuven, Belgium
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JA Verschakelen
###dept of radiology UZ Leuven, Leuven Belgium
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AU Wells
¶¶¶Royal Brompton Hospital, London, United Kingdom
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GM Verleden
+++University Hospital Gasthuisberg, Lung Transplantation Unit, Leuven, Belgium
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Abstract

Pulmonary fibrosis is the end stage of many diffuse parenchymal lung diseases. It is characterised by excessive matrix formation leading to destruction of the normal lung architecture and finally death. Despite an exponential increase in our understanding of potentially important mediators and mechanisms, the delineation of primary pathways has proven to be elusive.

In this paper susceptibility and injurious agents such as viruses and gastroesophageal reflux and their likely role in initiating disease are discussed. Further topics that are elaborated are candidate ancillary pathways, including immune mechanisms, oxidative and endoplasmic reticulum stress, activation of the coagulation cascade and the potential role of stem cells. The paper will try to provide the reader with an integrated view on the current knowledge and attempts to provide a road map for future research.

It is important to explore robust models of overall pathogenesis, reconciling a large number of clinical and scientific observations. We believe that the integration of current data into a "big picture" overview of fibrogenesis is essential for the development of effective antifibrotic strategies. The latter will likely consist of a combination of agents targeting a number of key pathways.

  • Coagulation
  • fibrosis
  • immunology
  • pathogenesis
  • pulmonary fibrosis
  • ERS
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Vol 61 Issue 2 Table of Contents
European Respiratory Journal: 61 (2)
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The pathogenesis of pulmonary fibrosis: a moving target
Wim A Wuyts, C Agostini, KM Antoniou, D Bouros, RC Chambers, V Cottin, JJ Egan, BN Lambrecht, R Lories, H Parfrey, A Prasse, C Robalo-Cordeiro, E Verbeken, JA Verschakelen, AU Wells, GM Verleden
European Respiratory Journal Jan 2012, erj00730-2012; DOI: 10.1183/09031936.00073012

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The pathogenesis of pulmonary fibrosis: a moving target
Wim A Wuyts, C Agostini, KM Antoniou, D Bouros, RC Chambers, V Cottin, JJ Egan, BN Lambrecht, R Lories, H Parfrey, A Prasse, C Robalo-Cordeiro, E Verbeken, JA Verschakelen, AU Wells, GM Verleden
European Respiratory Journal Jan 2012, erj00730-2012; DOI: 10.1183/09031936.00073012
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